We use cookies to distinguish you from other users and to provide you with a better experience on our websites. Close this message to accept cookies or find out how to manage your cookie settings.
We use cookies to distinguish you from other users and to provide you with a better experience on our websites. Close this message to accept cookies or find out how to manage your cookie settings.
Published online by Cambridge University Press: 01 June 2011
Localized iron overload can sometimes occur in the lungs (pulmonary hemosiderosis) and the kidneys (renal hemosiderosis).
Pulmonary hemosiderosis
Idiopathic pulmonary hemosiderosis (IPH) (also known as Ceelen–Gellerstedt syndrome) is a rare disorder of unknown etiology characterized by recurrent episodes of diffuse alveolar hemorrhage and accumulation of storage iron in the lung parenchyma. It is most common in children (age 1 to 7 years) but can also occur in adults. Clinical manifestations of IPH include pulmonary symptoms (hemoptysis, dyspnea, cough), parenchymal lesions on chest X-ray, and iron deficiency anemia of unknown cause. Diagnosis depends on exclusion of other disorders, such as inflammatory pulmonary capillaritis, in which diffuse alveolar hemorrhage is a cardinal sign. The clinical course of IPH is variable; in chronic cases, the localized iron overload can result in pulmonary fibrosis, and death can sometimes occur due to pulmonary hemorrhage. Treatment includes supportive therapy and administration of corticosteroids that can be combined with other immunosuppressive agents, such as azathioprine. Successful resolution of some cases of IPH with immunosuppressive drugs suggests that an immunologic mechanism could be involved in the pulmonary capillary damage underlying alveolar bleeding which, in turn, leads to pulmonary iron accumulation.
Hematite miners and other workers chronically exposed to iron ore dust may develop iron overload of the lungs and adjacent lymph nodes, but serum iron measures are usually normal.
Renal hemosiderosis
Marked iron accumulation in the kidneys is rare in hemochromatosis, but when present, storage iron is usually located in cells of the tubules, particularly the convoluted tubules.
To save this book to your Kindle, first ensure [email protected] is added to your Approved Personal Document E-mail List under your Personal Document Settings on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part of your Kindle email address below. Find out more about saving to your Kindle.
Note you can select to save to either the @free.kindle.com or @kindle.com variations. ‘@free.kindle.com’ emails are free but can only be saved to your device when it is connected to wi-fi. ‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.
Find out more about the Kindle Personal Document Service.
To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Dropbox.
To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Google Drive.
